Syndrome of encephalopathy and fatty degeneration of viscera in New Zealand children.

In 1963 a new syndrome was described in Australian children based on the association of severe fatty degeneration of the liver with acute encephalopathy (Reye et al., 1963).Twenty-one children in Sydney had an acute illness with profound disturbance of consciousness progressing to coma, and, in the majority, to death within two and a half days of admission to hospital.Prodromal respiratory symptoms, persistent severe vomiting, disturbed respiratory rhythm, convulsions, altered reflexes and muscle tone, and hypoglycaemia were often present.Pronounced cerebral swelling and fatty changes in the kidneys were additional findings at necropsy.In the correspondence which followed this first description similar cases were reported from England, Scotland, South " Acidosis " blood urea Pathological Intense diffuse fatty Fatty change in renal Fatty infiltration of change in liver.tubules.myocardium.Cerebral swelling.Reactive changes in Gastro-intestinal Absence of specific lymphoid tissue haemorrhage findings